Uropathology

2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran

Juxtaglomerular Cell Tumor of the Kidney

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4835
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Definition

Juxtaglomerular cell tumor is a rare neoplasm secreting renin. It arises from the specialized smooth muscle of the glomerular afferent arteriole in the juxtaglomerular apparatus of the kidney.

Clinical Features

  • Incidence

    Juxtaglomerular cell tumor is very rare with approximately 100 cases reported in the literature.

  • Age

    The tumor typically occurs in young adults with a peak incidence in the second to fourth decades.

  • Sex

    There is a slight female predominance.

  • Treatment

    Partial or radical nephrectomy is the standard of care.

  • Outcome

    Because the tumor secretes renin, the patients present with severe and poorly controlled hypertension, hyperaldosteronism, and hypokalemia. Usually, the clinical symptoms resolve after surgical removal. These tumors usually have an indolent course.

Macroscopy

Juxtaglomerular cell tumor is a yellow-tan mass circumscribed by a fibrous capsule.

Microscopy

The tumor is composed of uniform polygonal to spindle cells with central round uniform nuclei and...

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References and Further Reading

  1. Capovilla, M., Couturier, J., Molinié, V., et al. (2008). Loss of chromosomes 9 and 11 may be recurrent chromosome imbalances in juxtaglomerular cell tumors. Human Pathology, 39, 459–462.CrossRefGoogle Scholar
  2. Kim, H. J., Kim, C. H., Choi, Y. J., et al. (2006). Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: Report of 5 cases. Archives of Pathology & Laboratory Medicine, 130, 707–711.Google Scholar
  3. Kuroda, N., Maris, S., Monzon, F. A., et al. (2013). Juxtaglomerular cell tumor: A morphological, immunohistochemical and genetic study of six cases. Human Pathology, 44, 47–54.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly